Tuesday, December 22, 2009

I couldn’t sleep thinking about this “cure”. I was wondering is there is a rush to yell cure?

Researchers gave 10 patients ages 16 to 45 bone marrow Stem Cells. They gave them low levels of radiation, chemotherapy and high levels of immune suppressant drugs.

People with SCD normally have spleen problems; this makes them more prone to infection. Think about the infection possibilities when you give a person, who’s already catching everything that blows by, an immune suppressant drug. You may have received a cure for sickle cell, but now you may get cancer to replace it.

Researchers said, “Though most patients in the study are still taking immune-suppressant drugs, researchers hope to eventually wean them off the medications.” Check out the side effects of Alemtuzumab, a drug used to suppress immune system T-cells, and Sirolimus, an immune suppressant to fight rejection.

Let’s not run to the hills crying CURE until you can give a person a better quality of life, not replace their problem with another one.

Tuesday, December 15, 2009

The airways are popping. The media blitz is in full affect. Massive editorial posts have been sent all over the internet. The word is out. There MAY be a cure for Sickle Cell Disease.

According to a New England Journal of Medicine report dated December 10, 2009, researchers at the U.S. National Institutes of Health say that a new method of bone marrow transplantation cured nine out of 10 adult patients with sickle cell disease.

NEW…hmmmm. I’ll take a big mac, fries and a bone marrow transplant…. please.

Here’s what I’ve gathered from reading the media stuff.

Old Bone Marrow Transplant Method - In conventional bone marrow transplants, high doses of chemotherapy drugs and radiation were given. Chemotherapy and radiation is used to wipe out the person's own bone marrow, which makes the faulty red blood cells. There are many complications including destroying fertility

Adults were usually not good candidates for bone marrow transplants because they were thought to be too sick to handle the high doses of chemotherapy and radiation needed to prep the body for the procedure.

New Bone (Stem Cell) Marrow Method - Senior study author, Dr. John Tisdale, a senior investigator at the U.S. National Institutes of Health explained the new method allows for less grueling pre-transplant routines, which adults with severe sickle cell can tolerate.

10 patients ages 16 to 45 with severe sickle cell disease received bone marrow from donors that were siblings with matched HLA (human leukocyte antigen) in their blood.

Lower levels of radiation were used in the new method which does not seem to destroy fertility. The bone marrow is replaced with stem cells from a donor's marrow, which then takes over and begins to produce new, healthy red blood cells.

NOTE: When doing the new bone marrow transplants, the researchers noted that not all of the patient's own marrow was wiped out. Some remained and seemed to co-exist with the donor marrow without causing problems. Dr. Tisdale said, "That meant we didn't necessarily have to kill the entire bone marrow of the patient to make this work." Tisdale said, this opens the possibility of using an even less toxic means of preparing the body for transplant.

After 30 months, all 10 patients are alive, and nine of the patients had successful grafts where none of the patients experienced graft-versus-host disease (where the body rejects the new bone marrow). They are also considered cured of sickle cell disease, according to the study. Though most patients in the study are still taking immune-suppressant drugs, researchers hope to eventually wean them off the medications.

The new procedure doctor’s say is promising, especially since it could eventually include those who don't have an HLA-matched sibling. Dr. Tisdale said, "These were the sickest of the sick patients. Some were in the hospital every other week for pain or other crises. Today, some have gone back to school and to work. One patient had a baby."

In the past, these older/sicker patients were excluded from transplant studies as they are very poor candidates for high dose chemotherapy regimens. This study makes it possible to offer patients with severe sickle cell disease stem cell transplants."

******************************************************************************My conclusion: This is a “study”….also know as a “trial”. We won’t see this stem cell treatment given to “us” for a while. But, I’m hopeful.******************************************************************************

Monday, November 30, 2009

Not 100%, using one good eye, but living my motto "Live On". No pity party here, just information, education and support.

Now on to business.

Get Educated: The Department of Education is accepting applications for new SSS Program awards for FY 2010. The purpose of this program is to increase the number of disadvantaged low-income college students, first generation college students, and college students with disabilities who successfully complete a program of study at the postsecondary level.

Eligible applicants include institutions of higher education. Application deadline is December 7, 2009. Check out these sites for more information:

Tuesday, October 20, 2009

I wrote this in response to losing my eyesight for two weeks.When your body fails you fear will steps in. During this time you may ask yourself, “How will I make it?”, or “Who will help me?” and “Why do I have to suffer?” After this, pity comes, and then depression. You might then ask yourself, “Is life only about suffering?”

To all of these questions there is no correct answer. Suffering is a reality of life that brings out the best and the worse of us all. We become stronger from the pain or we break from the pressure. Both a blessing and a curse, suffering sheds light on our strengths and expose our weaknesses.

In all of this, I am a witness to both suffering and joy, both fear and fearlessness. Life, in one way or another is not fair and full of pain. It is scary and we don’t know the ending to our individual stories. Added to this, fear of the unknown waits to disturb our hearts. It limits our lives from enjoying what we do have. What I have concluded is that life is hard, get a helmet! And, that fear is useless.

Past all of my suffering is surrender. In that place of surrender, where I choose to live, I smile and thank God for each new day. In that place, beyond sickle cell disease and all of its limitations, I find peace and rest. This is where I can still sing, dance and enjoy the goodness of God’s blessings. I rest in God, wipe my tears and recover from my sufferings.

Finally, I have to agree with Saint Theresa who wrote, “Let nothing disturb you, let nothing frighten you. All things pass away, but God never changes. Patience obtains all things and he who has God finds he lacks nothing. God alone suffices.”*

Tuesday, September 15, 2009

There are things in the lives of people with sickle cell disease that trigger a sickle cell “crisis”. Some things like simply living, working and surviving can causes triggers to push us over the edge into the pit of pain. Other things, in some cases, are preventable like lifestyle choices. Either way, once the trigger is set, a sickle cell crisis begins with full fury.

Once the crisis has begun, usually a hospital stay follows. God forbid a crisis leads to death, but usually a blood transfusion, oxygen, antibiotics, pain medication, and glucose (for hydration) are needed. After a week, maybe two, in the hospital you come out feeling thinner, slower, and hoping to never go back again. Wishful thinking.

Since we’re living in a stressful world full of every kind of trigger, it is important for people with Sickle Cell to really take care of themselves. What we can’t control, we should let go of. What we can control, we should. Good living and good habits will help us live.

Ps. I want to live as long as possible…so, I’m taking a nap right now! ;-)

Tuesday, September 8, 2009

As a child I wondered why minor cuts to my legs and arms took a long time to heal. Everyone thought I picked or bothered my injuries but I hadn’t. They just wouldn’t heal. Sometimes, they would get infected and spread. As a child, I didn’t know what was wrong with me. Now I do. People with Sickle Cell Disease sometimes get leg ulcers that don’t heal. The reasons why are listed below. Live and learn.

What Causes Leg Ulcers?

Years of research have shown that the usual causes of leg ulcers are not a problem with the skin itself, but the lack of blood flow to the skin.Because blood supply to the skin is crucial, ulcers can be a result of poor circulation. They can also be a result of chronic medical conditions such as diabetes, obesity, rheumatoid arthritis and hypertension (and sickle cell disease). Ulcers are also commonly associated with depressed or lowered immune systems and people who’ve had their spleen removed.

Treatment for leg ulcersWhen treating leg ulcers, there are several areas that should be considered. Treatment can be a combination of: antibiotics, wound or ulcer care, anti-inflammation bandages, and elevation.Once an ulcer develops, there is always the risk of infection due to bacteria getting into the ulcer. Antibiotics may need to be prescribed to control the bacteria growth and the spread of any infection.

Wound cleansing for the treatment of infection and increasing circulation are crucial. Many ulcers need weekly wound care to help them heal. This consists of having the ulcer regularly cleaned to remove any debris and rebandaged with clean bandages.

Anti-inflammatory treatment includes application of bandages wrapped with several layers of bandages over the dressing, and then a compression stocking is placed over the bandages. This helps to keep the pressure down in the veins, which is thought to be the most important aspect of ulcer healing. If any numbness or problems with circulation are discovered bandages should be removed immediately.

So long as there is no arterial disease, leg ulcers will also benefit from elevation. Elevating the leg higher than the hip helps to pull any fluid that has collected in the direction it should go, (towards the heart). This helps prevent excess swelling in the leg, which helps to relieve excess pressure in the veins of the leg as well, and promotes better healing.

If ulcers are neglected (because they don't cause pain) or if ulcers are not treated, they can lead to more serious problems.

PreventionOnce the causes of leg ulcers are under control the ulcer should heal by itself. Successful prevention for leg ulcers and successful treatment for leg ulcers must be directed at correcting the underlying cause, or what has prevented healing, not the ulcer itself. If an underlying disease is one of the causes of leg ulcers (diabetes, obesity, rheumatoid arthritis and hypertension (and sickle cell disease), it's important that it is treated.

Leg and foot ulcers have a tendency to recur in elderly people, and sometimes may require years of therapy. Prevention may include weight loss (if you are overweight) and regular exercise to promote good circulation. Body Detox can also help to purify the blood and cleanse the system. Good nutrition is also a good way to increase the body’s natural defenses to prevent ulcers.

Smoking is known to decrease the amount of oxygen that gets to the ulcer through the bloodstream, which prevents potential healing. If you suffer from a leg ulcer and smoke, you should stop smoking to allow better blood flow to the wound.

NutritionNutrition plays a big role in healing leg ulcers. Getting the right mix of treatment and nutrition can help an ulcer heal. There are certain vitamins and minerals that are known to aid in the healing process: vitamin C is one of these vitamins. It has been found that vitamin C mixed with bioflavonoids increases the potential of better circulation.

Other vitamins known to help with circulation are vitamin E, B complex, zinc and amino acids. Vitamin K is also important; it is known to play an important role in the clotting of the blood. Getting the right nutritional balance with vitamins and minerals helps to fight infection, aids in blood clotting factors and properly oxygenates the blood for faster healing.

Your diet also plays a big role in curing a leg ulcer. A diet high in fiber and a lot of leafy, green vegetables, especially dark green vegetables, is best. You should avoid sweets and refined sugar and eat more garlic and onions, which help to increase circulation.

SurgerySometimes, it is necessary to perform surgery to help a leg ulcer to heal. It is a procedure that requires disconnecting malfunctioning perforator veins that are not allowing proper blood flow to get to the extremity. With the disconnection, the blood flow is redirected through the good veins and can get to the ulcer more efficiently to aid in healing it. After surgery, it is important to get plenty of rest while keeping your leg elevated. You will also need to continue wound care and to keep the ulcer free of bacteria.

Monday, August 31, 2009

I surveyed a group of people who suffer with Sickle Cell Disease. The list of major issues they face with management of their disease AND with their interactions with healthcare providers are listed below:

1. Don’t like the side effects of medications (i.e. MS Contin, Vicodin).2. Lack of compassion from healthcare providers.3. Doctor’s who reduce pain medication prescriptions, not believing their pain levels.4. Hospitals have a rule “3 doses of pain medicine, then discharge.” Not fair!5. Health care professionals have nonchalant attitude regarding pain (no compassion).6. When experiencing pain they are under medicated.7. Teaching healthcare clinics seem to treat patients better, more informed about pain.8. Discrimination by white doctors regarding SCD disease…demeaning treatment.9. As tolerance to pain medicine increases, healthcare professionals debate increasing dose or quantity.10. When experiencing a sickle cell “crisis”, they are under-treated. Pushed out of hospital before fully well.

I'm appalled just reading this list. I've experienced each and every one of these issues, but it makes me mad to see that others have too.

People who are sick, are sick...believe them. Most people who suffer with sickle cell disease only want help. They're not "happy" about being in pain just to get drugs. Believe that too!*

Tuesday, August 18, 2009

Blood is the life force within our bodies. Like oil in an engine, we can’t run without it. We can live with a transplanted heart and we can live without a kidney. If we lose a limb, we can order up a prostatic part and keep going.

Blood, however, cannot be replaced entirely. Sure, we can get transfusions, which will supplement what we already have, but we need a full supply to live.

People with sickle cell have red blood cells with a shorter life span. Usually, red blood cells live 120 days. For people with sickle cell the red blood cell lives only 10 -20 days. When those short-lived red blood cells die prematurely it also causes anemia. To top that off, in addition to having short-lived red blood cells, the ones we do have are sickled shaped and lacking oxygen.

Transfusions may be needed if red blood counts get too low to sustain life. Blood is made up of red blood cells, white blood cells, platelets, and plasma. Blood is transfused either as whole blood (with all its parts) or as individual parts. Every person has one blood type (A, B, AB, or O). Also, every person’s blood has an RH negative or RH positive. When getting a transfusion, the blood type received must work with your blood type.

*

Getting many blood transfusions can cause too much iron to build up (iron overload). People, who require multiple transfusions, are at risk for iron overload. Iron overload can damage the liver, heart, and other parts of your body. Blood transfusions can also damage lungs, making it hard to breathe. Most patients recover, however, 5 to 25 percent of patients (who are very ill to begin with) develop life threatening lung injuries.

When getting blood transfusions there is a (small) chance of contracting a virus or infection. In addition, some people experience allergic reactions. (I once grew hair on my chest after receiving a transfusion.) Hey, I’m a woman and this ain’t supposed to happen.

At some point, most people with sickle cell disease will need a blood transfusion, some more than others. Overall, this life flowing fluid is a blessing. Those of us who receive it, thankfully do. Those of you who give it, thank god for you.

Monday, July 20, 2009

The first time I heard about football players (with sickle cell) dying on the field was a story about a young player from Florida. I read, after he died from heat and over exertion, his family was blamed for "not telling anyone" that he had sickle cell trait. The problem was, he nor his family knew that he had sickle cell.

This terrible situation may be prevented forever, according to CBS Sports.com Senior Writer, Dennis Dodd. In his June 29, 2009 article, NCAA to recommend schools test for sickle cell trait he states, ... the NCAA and Rice University settled a lawsuit brought by the family of a Rice football player (Dale Lloyd) who died in 2006 due to complications resulting from sickle cell trait.

As a result of the settlement, the NCAA, regarding player safety, will now recommend testing for sickle cell trait of its players. In addition, Rice will help sponsor NCAA legislation to make sickle cell trait testing mandatory

Dale Lloyd did not die in vain. The legacy he leaves behind will benefit football players all over America. They will be tested prior to those HOT summer workouts and maybe now, they won't blame the players for not knowing.

Wednesday, July 8, 2009

When you are in pain the last thing you want to do is move your body. When a person with sickle cell disease has a episode of pain or “crisis, ” the pain is beyond understanding. What I have discovered is if I strengthen my body through exercise I am better able to manage pain. This is not to say exercise prevents a crisis or helps during a crisis. It is to say, a strong body is a healthy body.

Here’s my exercise regime. Three days a week: I stretch my legs to get ready for exercise, I walk for 30 minutes, then lift I light weights (3 or 5 lbs). To end it all, I gently stretch my arms, legs, neck, and back (somewhat like yoga stretching). After this exercise I feel strong and balanced. Of course, I eat well too….fruits and vegetables, and lean meats.

People with sickle cell disease have to participate in their own care. We have to take responsibility for what we CAN do to improve our health, and then let our medical professionals help us too. Keeping our appointments for preventive care; like mammograms, bone scans, blood test, eye exams, and dental exams all play a part in helping us live well.

Sunday, June 21, 2009

Order a copy of the magazine at http://www.abilitymagazine.com/. You can order a PDF (electronic) copy of the issue for $2.95 or you can have it mailed to you for $6.95. Kristi Yamaguchi is on the cover of the April/May 2009 issue.

This article confirms my mission. I have determined to cry out to the world about living with Sickle Cell Disease. Also, for the sake of patients all over the world, I want to bring more attention to the suffering associated with this disease.

Thursday, June 18, 2009

I’ll start by saying I’ve had two non-serious strokes and many silent ones. It seemed odd to me that while I was having those strokes, doctors who treated me never wanted to say the word “stroke.”

I had all the symptoms: blurred vision, slurred speech, intense headache, and numbness on face and hands. Doctor’s seeing these symptoms treated me for pain, worked to bring my blood pressure down, and performed ultrasound scans to see if any clots existed, but they never said “You had a stroke.”

Thankfully, I’ve recovered. I also found out that people with blood disorders (like Sickle Cell) are high risk for having strokes. See more information below, get informed and live.

What is a Stroke?

Blood is circulating through your body all the time in arteries and veins. Usually, these blood vessels work fine and there's no problem. A stroke can happen if something keeps the blood from flowing as it should. A person might have a clogged blood vessel, or a blood vessel may have burst, flooding part of the brain with blood. Either way, with a stroke, brain cells die because they don't get the oxygen they need.

A "Mini stroke" or “silent stroke” is not full-blown stoke because the blood flow is only cut off for a short time and they don't cause the same kind of damage right away. These mini strokes are a warning sign that something is wrong and that a real stroke may be on its way.

What Happens During a Stroke?

A stroke usually happens suddenly, and a person having a stroke has several signs:

numbness or weakness on one side of the bodya very bad headachedizzinessloss of balance or coordinationtrouble talking or understanding what people are sayingtrouble seeing

A stroke can cause learning disabilities and/or lasting brain damage, long-term disability, paralysis (an inability to move), or death. Anyone who has even one of these symptoms should get to the hospital right away.

What Are the Risk Factors?

Stroke occurs in all age groups, in both sexes, and in all races in every country. In African Americans, stroke is more common and more deadly--even in young and middle-aged adults. Conditions linked to stroke are:

Recovering from a stroke can happen quickly or can take a long time. How soon someone gets better depends on how bad the stroke was and how healthy the person was before the stroke. People who have had a stroke may need medicine or surgery. Later, they may need re-habilitation. Treatment for a stroke will depend on what caused it.

Medicine - For a stroke caused by a clogged blood vessel (ischemic), the doctor might give the person medicine that thins the blood and keeps it from clotting too much.

Surgery - Doctors may do surgery to open up a clogged blood vessel to help prevent another stroke later on. If a person has had a hemorrhagic stroke, surgery may be needed to remove blood clots or fix weak blood vessels.

Some strokes can be prevented in adults. Stroke-prevention tips are: Don't smoke, Don't drink too much alcohol, Eat healthy and be active. This can help lower cholesterol. Check blood pressure. High blood pressure is a major cause of stroke. Don't ignore problems like heart disease, high cholesterol, or diabetes.

Tuesday, June 2, 2009

The United Nations (UN) celebrates the first annual Sickle Cell Disease World Day at the UN in New York.

I can't help being happy, excited and overjoyed. The United Nations General Assembly approved a Resolution proposed by the delegation from the Republic of Congo Brazzaville and co-sponsored by 24 Member States.

June 19th of each year was established as Sickle Cell World Day at the UN.

This years activities include: Testimony Stand exhibitions by patients' organizations and international organizations, an art exhibition on the disease, panel and round table discussions, a First Ladies and Royal Highnesses meeting, and a gala dinner.

In partnership with UNICEF, the World Health Organization (WHO) and the UN, this Sickle Cell Disease World Day event will bring global awareness to Sickle Cell Disease and its growing public health problem.

If you're in the New York area (or can get there by plane, train or automobile), please get more information and support this worthy event.

Tuesday, May 26, 2009

Picture this, you have Sickle Cell Disease (a disease of the red blood cell) and you marry a man who is diagnosed (at age 44) with Beta Thalassemia (also a disease of the red blood cell). Now you think to yourself; why didn’t this man know he had a blood disease?

Often people who have Thalassemia don’t know they have it. Sometimes they’re told, “You’re anemic,” and that’s all they’re told.

Thalassemia is similar to Sickle Cell Disease. It is also a genetic defect of the hemoglobin within the red blood cell. Thalassemia, however, is the defect in the number of hemoglobin made in the blood, where Sickle Cell is a defect of the hemoglobin (sickle shape). Either or both of these conditions cause anemia. Some people may have both of these conditions at the same time.

This is where my utter shock comes in. My husband was told by his parents that he was anemic, but at 44 he was officially diagnosed with Beta Thalassemia. My husband is African American and the doctor who diagnosed him is Chinese. The doctor informed him that they have “the same type of Thalassemia.”

Are you getting this? A Chinese doctor and an African American man have the same blood disorder.

There are two issues that come to mind when I remember this story. Firstly, this is another example of my main argument that blood disorders are diseases of humans, not of one race or another. And, secondly, I see the need for better testing/education practices. People should know what their health limitations are, and in result, understand the impact on them having children.

People living with Thalassemia suffer just like people living with Sickle Cell Disease. Just imagine a child of ours having Beta Thalassemia AND Sickle Cell Disease. Luckily, we do not have any children, and thank God I didn’t have to find out what that would be like.

Tuesday, May 12, 2009

I was recently sent an email where I was asked my opinion regarding sickle cell management in the US and abroad, as it relates to improving care for sickle cell disease. The question was posed by suggesting more of a cultural/social model of care rather than a medical one.

I am just a patient with Sickle Cell Disease, and an advocate for people living with the disease. I am not a medical professional, I am not an expert, nor do I profess to know what a “medical model” is verses a “cultural/social” one. All I know is my experience with having Sickle Cell Disease.

As a child, I received treatment by medical professionals within my “cultural” community and the care I received was gentle, compassionate and well trained. It was not until I grew up, began to travel and moved to different communities. More than once I’ve been told by uninformed medical professionals, “What do you have? Sickle What?”

I was disgusted when doctors would tell me, “Oh, you have Sickle Cell, only black people get that disease.” Or, doctors would say, “Oh, we don’t get many black patients here, so we don’t see cases of Sickle Cell.” This calls for more training of medical professionals.

Just for the record, my niece looks white. She is ¾ Anglo & ¼ African American. Because we have a family history of Sickle Cell, she asked her doctor to perform an electrophoresis test to see if she was a carrier for Sickle Cell Disease. He told her, “You’re white, you don’t have that disease.” She insisted, because of her family history (her mother has it). Low and behold, was tested and found to have the Sickle Cell trait. So much for “you’re white, you don’t have THAT disease.” Again, this calls for more training of care providers.

For this very reason, I believe Sickle Cell care should be approached in a patient centered model; (as a disease of humans), not as cultural model (a disease of one racial group). Sickle Cell Disease is creeping silently into all populations, all races (because of mixing) and all cultures. Now, that’s my opinion.

Wednesday, April 22, 2009

When you go to the ocean the altitude level is equal to zero (0). When you travel up a mountain the altitude level increases and the air pressure decreases. At places like the Grand Canyon the altitude goes up to over 6,000 feet elevation, and in the Rocky Mountains of Colorado it can peak to over 14,000 feet elevation. As the elevation increases, the altitude is higher and the air pressure is lower. The lower the air pressure, the lower the number of oxygen molecules available to breathe. This is also known as thin air.For people with Sickle Cell Disease, less oxygen means SICKNESS.

My altitude sickness symptoms begin around 3,000 feet. It starts with a headache, feeling tired, and dizziness. If I don’t get out of the mountains, then all of these symptoms get worse. At 4,000 – 5,000 feet, I begin to get confused and agitated. I have shortness of breath, my spleen, hands and feet begin to swell, and my head aches with no relief. At 6,000 feet, I descend into needing hospitalization. I throw-up, more headaches, dizziness, lips and palms turn white, and my body slowly collapses into a full Sickle Cell crisis.

How do I know so much? I found out about altitude sickness by accident. After taking a few vacations to the Grand Canyon and Utah I experienced altitude sickness first hand, the hard way. I travelled to these high places, got sick, and learned my lesson. I discovered that I can not go higher than 4,400 feet elevation EVER (without an oxygen tank). In winter, because oxygen is thinner by nature, I can not go above 3,000 feet elevation.

People with Sickle Cell disease have physical limitations, as well as geographic limitations. I know that I can’t visit the Swiss Alps, unless I have an oxygen tank strapped to my back and a mask flowing cool, clean oxygen into my lungs. That's ok with me. I am informed about my limitations and I can accept them. Information is power.

Now take a minute to inhale, now exhale. Enjoy each and every breath you have because it's very precious.

Sunday, April 5, 2009

Some people have asked why I posted two previous blogs from a health-care worker in India?

Well, in my opinion, Sickle Cell Disease and Thalassaemia are global problems that are growing. Ignorance and avoidance have limited the recognition that these disorders have a true impact on society. With global immigration increasing, we have to look at the global impact these diseases have on children, healthcare, healthcare cost, and education worldwide.

Currently, there are American voices crying out for people suffering with these diseases, and they are getting things done in America. Thank God. There are no global voices, however, telling the worldwide story of pain, suffering, and health-care limitations related to these two diseases.

As Sickle Cell Disease and Thalassaemia are silently transferred from one generation to another, all voices are important. Hari Prabhakar, from the Sickle Cell Disease Center at Gudalur Adivasi Hospital in India offers his voice, and I gratefully listen.

********************************************************************Working at the Sickle Cell Center at Gudalur Adivasi Hospital over the past 5 years has taught us a wide variety of things from both a patient and provider perspective.

Existing literature and health authorities continually stress the importance of cultural sensitivity and community consensus as a means of developing an effective healthcare management system. We have certainly found this to be true, but still face challenges in the implementation of effective healthcare programs and must overcome obstacles such as:

Providing awareness of the disease in the Adivasi population - This is extremely difficult when dealing with a population with a strong and widespread belief system. We use members from the Adivasi community as key health educators (in the villages) to help in gaining acceptance and support for the program. However, a lot more needs to be done regarding genetic counseling managed internally and that does not offend Adivasi group’s beliefs.

Confidentiality of test results - Given that awareness of the disease and its causes is still unfamiliar, there exists a fair amount of shame when an individual is labeled with the disease. It is interpreted that a curse is upon them. Cards are given out to document test results of individuals and are coded to be interpreted only by healthcare providers and patients. Explaining blood test results is also the responsibility of an Adivasi health worker, who we feel can best explain the disease to the patient and instruct them on the appropriate course of action.

Getting medications - Medications like Hydroxyurea (a rather expensive cancer drug used for the treatment of SCD) is hard to get in developed countries where there are areas of geographical isolation. Transportation is too difficult or the patients are migrant workers with little time to take care of themselves. We have tried to stock supplies of critical medications like Hydroxyurea in area Centers throughout the Gudalur valley, where patients can also get their blood tests taken. And, a Sickle Cell Disability Fund is in place to help cover patient transportation costs and make up for lost time in an already unsteady employment situation.

Funding is always a major problem - With the prospects of offering “free” treatment to Adivasi patients and inpatient treatment for complications, we pursue public-private partnerships, private donations/grants, and cross-subsidize from non-Adivasi patients to ensure equity and access in services. Community-based insurance for SCD is also a possibility in order to ensure long-term sustainability and empower patients to become an active part of the financing process.

Researching and developing cheaper more sustainable medicines - In a resource-strained environment where other basic public health needs are minimal, it raises to question the use of Hydroxyurea, the expensive tertiary-care cancer drug. As a result, we have team working to develop a plant-based anti-sickling agent that may be cheaper than Hydroxyrea and more sustainable to produce. In Nigeria, for example, the plant-based drug, Ciklavit, has been shown to have positive results, and we hope that have something similar which could be used locally and internationally.

So, what can we learn for use in the United States?

Regardless of whether we are dealing with a rural village or sprawling metropolis, developing skills of health workers to serve as liaisons to the community is important in gaining support and acceptance. A health service with little confidence from the public will ultimately fail.

There certainly needs to be a push for greater training in SCD among health workers, many of whom could be chosen from the local community to conduct education programs, promote counseling, etc… Complete coverage of the SCD population in the US should be done in tandem with local communities.

Most importantly, however, listening to patients and determining their needs, capabilities, and limitation will play a crucial role in expanding the role of sickle cell programs in the US into a wide-ranging program rather than top-down vertical program.********************************************************************

( HARI……. Thanks for your contributions to this blog and your suggestions.)

Monday, March 23, 2009

There is a lot of money to be made by non-profit organizations that support specific “diseases.” Patients, with those diseases, may or may not benefit from those organizations unless they seek them out. One thing that every patient with Sickle Cell disease can benefit from is the donation of blood.

I recently read an article about a young girl with Sickle Cell disease who has received over 400 blood transfusions in her short lifetime. She is now working with community churches to encourage donations as part of “Sickle Cell Sabbath.” This effort was directed to community churches because they are a symbol of goodwill and a pillar God’s love. Together with a local blood center, this young girl has begun a quest to ask churches for donations of blood, rather than money. A donation that we all know will be put to good use.

We can all learn a lesson from this young girl, who has moved past her personal struggle to enhance the lives of others. We can find creative ways to help people and meet their needs, as we care for our own. We can give money, if possible, and we can give love, always. We can also encourage the world to give blood and make a donation to someone’s life. xoxoxox

Tuesday, March 10, 2009

This post is part 2 of a contribution from Hari Prabhakar, from the Sickle Cell Disease Center at Gudalur Adivasi Hospital in India.

The recent 2007 World Health Organization (WHO) and Thalassemia International Federation (TiF) report (see link below) speaks on the need for comprehensive sickle cell centers in developing countries to manage the burden of SCD, and also notes that in the USA, existing centers only cater to 18,000 of the 80,000 affected. In India, there are around 5 million carriers and up to 20 million affected, and most of those affected, primarily Adivasis, live in areas with little access to basic health facilities.

In the south Indian state of Tamil Nadu, in the Gudalur Valley of the Nilgiris District, there is a tribal (Adivasi) population of over 25,000 who have lived in the area for thousands of years. Until 1913, there was a high prevalence of malaria, which could explain why the sickle gene and sickle cell disease has persisted for so long. Within the community, historically mysterious deaths and suffering were regarded as “Doshams” or curses. There was almost no awareness or concept of genetic diseases.

In the past, hydration therapy and vitamin supplementation remained the primary means of dealing with patients at Gudalur Adivasi Hospital. The hospital has doctors, nurses, and health workers, a large percentage of whom are Adivasis trained from the community. Unfortunately, we didn’t have a good idea of the prevalence of the disease in the area.

After reviewing some of the existing literature, doing some internal studies, securing startup funds, and talking to some hematologists, we launched the Sickle Cell Disease Center at the Gudalur Adivasi (Tribal) Hospital. The platform of our Center, now 5 years in existence, has been to emphasize screening, treatment, education, and research, and to serve as a national model for SCD nationwide. Realizing the cost of management of the disease would be out of reach for the population, we decided to offer all services free.

The first step in ensuring the effectiveness of the program was to bring awareness of sickle cell disease to the local population. Thanks to the hospital’s longstanding presence and community outreach, we have been able to deploy the Adivasi health workers to educate, while also providing comprehensive services including Hydroxyurea, penicillin, and pneumococcal vaccinations. Unfortunately, the pneumococcal vaccine for children under 5 is not yet available, but thanks to public health professionals throughout the world, it will be available in India for our patients soon.

Every month, there are screening camps in the villages to identify patients and carriers, as well as presentations to villages on SCD, led by our health workers. Those identified as patients are brought to the main Center in Gudalur and given appropriate treatment according to their needs.

In the summer months, the monsoon season hits the area and make transportation a tremendous issue. The terrain and lack of roads in the area make it difficult for those who need to come to the main Center for picking up their medications and getting monthly tests. We have stocked the appropriate medications in 8 area Centers of the Gudalur area to enhance patient accessibility

As of now, we have screened around 6500 and have 185 patients, many of them who are non-Adivasi but come from a community called the Chetty’s who also have a high prevalence of the disease. There are still thousands more in the villages who have not been screened and are undetected, and we hope to reach them in the upcoming months and years. Genetic counseling is certainly an important part of the program, and we are working on deploying a Center and field-based program that is culturally sensitive and appropriate.

Friday, March 6, 2009

I’ve heard too many stories about young people (ages 13-30) with Sickle Cell Disease who just give up on life. They believe they've gotten a bad deal from God, that life is unfair, and it's not worth living if it includes suffering.

In result, these young people drink alcohol, smoke cigarettes (and whatever else); and push their bodies beyond its limits. Trying to keep up with their friends, these young people end up in the hospital every time.

I know it’s hard to take care of yourself when you’re suffering and sad. I know that life seems unfair when you’re the only one with Sickle Cell Disease that you know. I know you want to live, like everyone else, and I also know how it feels to be 20 years old (with life ahead of you), and Sickle Cell dims your hopes for a future.

I have to tell you young people “Don’t give up!”

There is hope past the pain. There is life beyond blood transfusions. You’ve gotta just live. Live well and take care of yourself. I promise you there are happy days ahead….live to see them. There is wellness ahead….do your part to have it. There is a future for you....to do all the things you want (or can) do. Live to see it.

I tell you this (young people), from one SC sufferer to another, cry if you want to cry…then wipe your tears. Get up, dust yourself off and get on with your life. Go ahead and dream big dreams, and then do whatever you can to make those dreams come true. God will do the rest.

When you get weak and you fall, get up, dust yourself off and try again, and again, and again, and again, and again. Then, just smile, because you're not going to be defeated by anything…. not even Sickle Cell Disease.

Monday, February 23, 2009

This post is a contribution from Hari Prabhakar of the Sickle Cell Disease Center, at Gudalur Adivasi Hospital, India.

Sickle Cell Disease (SCD) is truly a global genetic disease, and there has often been a misconception that SCD is found only among those of African/African-American descent. In order to successfully manage the disease internationally and formulate global strategies, it is important that we understand the different types of challenges and lessons learned from sickle cell management from both the patient and provider perspective

As such, I would like to share with you our experiences in managing sickle cell disease amongst an indigenous population in South India, the lessons learned, and how we can use these lessons to improve SCD management in the United States.

Each year in India, 495,000 infants have congenital malformations, 390,000 have glucose-6 phosphorylase enzyme deficiency, 21,400 have Downs Syndrome, amongst the Adivasis population ((indigenous populations in India are also called Adivasis) 9,000 infants are born with Beta-thalassemia, and 5,200 have sickle-cell anemia. Due to inadequate diagnostic, management, and rehabilitation facilities, and lack of comprehensive knowledge/acceptance of the disease, the burden of these disorders among the Indian population is far greater than Western countries, and it is particularly pronounced among indigenous (Adivaisis) population in South India.

Adivasis, who are comparable to the Native Americans of the US, number around 10% of India’s population (around 100 million people!). They have been traced back to 3000 BC, and have genetic similarities to Australian aborigines and African tribes. SCD has the highest prevalence among this population, with up to 1 in 5 people being a carrier, and up to 1 in 20 having the disease.

As such, millions in India suffer and succumb to the disease, with little money, knowledge of the disease, or access to healthcare facilities to seek care. Due to a combination of social neglect, inadequate healthcare infrastructure and research, and lack of grassroots implementation and national policy, there are almost no comprehensive sickle cell programs in India, where it is needed most.

Much like in the United States, it is of utmost importance that those in India suffering from the disease are provided a voice and capability to seek care from one of the most despicable and significant genetic diseases on the planet..

Wednesday, February 11, 2009

Calling all people with Sickle Cell Disease to participate in a research study.

The study is sponsored by Ms. Phyllis Bazen, MSN, FNP-C, a Doctoral Candidate at the University of Rochester School of Nursing. Ms Bazen is the Study Coordinator.

The purpose of the study is to explore the most common stressors that adults with SCD cope with and to explore the effects that these stressors have on mood and/or quality of life perceptions.

People with Sickle Cell Disease have many things that bring stress; pain, depression, hopelessness, etc. etc. etc. We have to live on despite these stressors and try to have a meaningful, productive lives...anyway.

The best way to contact Ms. Bazen is the web site contact page, her cell phone, email, or 1-800-464-8668 (in US) See card below for information.

If you decide to participate in this study, you will be speaking with Ms. Bazen who conducts a one-on-one private (confidential) phone interview. In this interview, Ms. Bazen will ask a series of questions related to living with Sickle Cell Disease and related stresses.

The outcome of this study is to gather data related to the stress that Sickle Cell patients endure and find ways to better treat them (us).

Saturday, January 24, 2009

I was researching Sickle Cell Disease around the world…I’m just a curious soul…. and what I kept finding (in different reports) was the same quote: “The origin of Sickle Cell Disease is Africa and primarily affects people of African decent.” I even read one (racist) report that said: “The African footprint in the world is Sickle Cell Disease.” I was baffled. I’ve heard of cases of Sickle Cell Disease in India, Saudi Arabia, Asia, United States, Spain, Italy, Mexico and Africa.

My belief is that Sickle Cell Disease is a human condition, not just an African condition. I got busy researching to see if my belief was correct. What I found was related to genes and human diseases located on the World Health Organization’s (WHO) website.

The source of Sickle Cell Disease, I found, is not the African footprint, but DNA (DEOXYRIBONUCLEIC ACID). DNA is the blueprint of life and has the instructions for making each and every one of us. According to the WHO report, “Pure genetic diseases are caused by a single error in a single gene in the human DNA.”

The report goes on to say, Sickle Cell Disease is one of many “monogenic diseases” resulting from modifications (errors) in a single gene that occurs in all cells of the body. These monogenic diseases affect millions of people worldwide, and scientists estimate that over 10,000 human diseases are known to be monogenic.

Thursday, January 15, 2009

In people with Sickle Cell Disease their red blood cells are dehydrated (they are thirsty for water). These cells are thirsty each and every day. Just imagine living in the Sahara desert and there is nothing but sand. Eventually, you will die of thirst, and you will die quickly. Well, that’s what happens to our red blood cells. They need water….everyday.

Dehydration in red blood cells, (according to American Society of Hematology) is due to increased loss of potassium, chloride, and water. (Yes, momma says “eat those bananas for their potassium too!!!”) By keeping hydration (water) in red blood cells they can survive longer. With longer surviving red blood cells in our body we will have more red blood cells (sickled or not) available for our beautiful bodies to use.

There has been research in trying to hydrate red blood cells with medication (US Institute of Health clinical trial), but this research has not fully been successful.http://clinicaltrials.gov/ct2/results?term=ica+17043&cond= %22Anemia%2C+Hemolytic%22)

There is hope, however, that we can do our part by DRINKING MORE WATER, and we can wait for researchers to do their part. This is one more bit of information that our doctors don’t tell us. Why we need water? Now, I’m thirsty!

Saturday, January 10, 2009

A complication of Sickle Cell Disease is a condition of the eye called Retinopathy. This condition occurs when the blood vessels in the retina become blocked, grow haphazardly or become leaky, and extra blood is trapped within the space between the eye lens and the internal membrane of the retina. This can damage the retina and cause retinal detachment.

Retinal detachment is a medical emergency. The detachment of the retina is painless, however, if not treated quickly, can cause permanent, partial, or total vision loss. Symptoms include: sudden appearance or increase in the number of “floaters,” (which are shapes that float in the eye and are seen in the field of vision), brief flashes of light in the eye, loss of the eye’s central or peripheral field of vision, a curtain appears to fall over part of the visual field, and sudden changes or blurring of vision.

To see, light must be able to pass through to the retina without anything getting in its way. You may not be aware of changes to the retina until your vision has been impaired. Distinguishing if blood is floating between the internal membrane and the retina's nerve fiber layer is not always possible, and this is why we must have our eyes screened every year.

My daughter suffers from this Sickle Cell complication and the suffering she endures is heartbreaking. Cataract surgery (at 14 years old), and vision loss is something she has tolerated. I watch her battle for healthy eyes with tears in my own.

The branch of medicine that is concerned with the diagnosis and treatment of eye diseases and conditions is ophthalmology.

Friday, January 2, 2009

When I was 10 years old a well meaning doctor bent down to my 3 foot height and told me very gently, “You know, you’re only going to live to be 30 years old.” I blinked and thought, “That’s a long time from now.”

Fast forward thirty-eight years and I’m 48 years old and still alive.

That doctor didn’t realize that he had given a 10 year-old child a death sentence. He could not have imagined that his prognosis would sit in the back of my mind as I turned 20, then 30 and then 40 years old. As I aged, I laughed at that doctor’s prediction, but secretly I prayed for God to give me more time.

As time passes, and I age, I discovered that Sickle Cell Disease would take its place as the most important thing in my life. The effects on my body have been gradual, yet undeniable. Regardless of how well I “take care of myself”, I have to pay attention to everything I do as it relates to my health.

Recently, I heard a young man with Sickle Cell Disease say “Sickle Cell gets better as you learn how to live with it.” Sorry baby, that ain’t true. Sickle Cell Disease doesn’t “get better.” The lack of oxygen, low red blood count and trapped sickling cells eventually takes its toll on your joints, internal organs and your life. This is not bad news, it’s the truth.

There is good news, however. There are things we can do to help ourselves live and age well. I call them “the five mandatory things.” They are; drink lots of water (to keep your blood fluid), take vitamins (to get extra nutrients), rest, rest, rest (to rejuvenate your aging body), eat healthy foods (anything that assist in blood development {I know I hate liver too}), and exercise (mild movement to keep the aging body mobile and to increase oxygen). These five things have helped me live past my doctor’s dooms day prognosis.

Does Sickle Cell Disease get better? No. Do people living with Sickle Cell Disease get smarter? Yes.

The smarter you are the longer and healthier you’ll live. Will you still have episodes of pain? Yes. But, the better you get at managing your disease (doing those five things) the better you will manage the “triggers” of Sickle Cell “crisis”. The more clearly you are about accepting your condition, AND your responsibility to live your best life in spite of it, the better life will be. There are some uncontrollable issues like stroke, and spleen infarctions that can’t be managed, but so what.

We can hope for a “complete” cure, and in the meantime, also live full happy lives. I’ve even heard of people with Sickle Cell Disease living to be 70 & 80 years old. (I haven’t met any of them yet, but I hope to join their club of survivors.)

Do I know a thing or two about living this life with Sickle Cell Disease? Yes, I do. All 48 years of it (and past that darn number 30!)